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AIM: Familial Mediterranean fever (FMF) is a monogenic autoinflammatory disease characterised by recurrent episodes of fever and polyserositis. Sacroiliac joint involvement is rare in FMF patients. The purpose of this study was to evaluate the demographic, clinical, laboratory and imaging findings of patients with FMF who developed sacroiliitis. METHODS: The files of paediatric patients aged 0-18 years who were followed up with a diagnosis of FMF were retrospectively reviewed. FMF patients with evidence of sacroiliitis on magnetic resonance imaging (MRI) were included in the study. RESULTS: Among 1062 FMF patients, 22 (12 males; median age 8.5) (2.1%) of them were found to have sacroiliitis. FMF was diagnosed before sacroiliitis in nine (40.9%) patients and after in 13 (59.1%) patients. The most common symptom in patients with sacroiliitis was low back pain (n = 21, 95.5%). In MEFV gene analysis, M694V was found in 16 (72.7%) patients and was the most common mutation. MRI showed evidence of sacroiliitis in all patients. All patients were using colchicine. Patients with FMF-associated sacroiliitis, remission was achieved with non-steroidal anti-inflammatory drugs in 12 (54.5%), conventional disease-modifying antirheumatic drugs in six (27.3%) and tumour necrosis factor inhibitor treatment in four (31.8%). Four (31.8%) patients experienced sacroiliitis when colchicine incompatible and four (31.8%) patients experienced sacroiliitis while using biologic agents for colchicine-resistant FMF. CONCLUSIONS: FMF-associated sacroiliitis should be considered especially in patients with M694V mutation if they have symptoms such as low back pain. Colchicine-resistant FMF patients should be evaluated for sacroiliitis symptoms at each visit.
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Objectives: This study aimed to investigate coronavirus disease 2019 (COVID-19) vaccination rates and factors affecting vaccination in children with rheumatic diseases. Patients and methods: This multicenter cross-sectional survey-based study was conducted between July 2022 and September 2022. Four hundred seventy-four patients (256 females, 218 males; median age: 15 years; interquartile range, 13 to 16 years) were included in the patient group, and 211 healthy children (124 females, 87 males; median age: 15 years; interquartile range, 13 to 16 years) were included in the control group. A questionnaire was administered to the parents face-to-face during routine outpatient visits. Results: Of the patients, 220 were followed up with the diagnosis of autoinflammatory disease, 174 with juvenile idiopathic arthritis, 48 with connective tissue disease, 23 with vasculitis, eight with uveitis, and one with sarcoidosis. In the study group, 256 (54%) patients and 115 (54.5%) healthy children received at least one dose of COVID-19 vaccine. Parents' concern regarding potential side effects of the vaccine was the most common reason for COVID-19 vaccination hesitancy in both groups. The median patient age, follow-up period, colchicine treatment rates, childhood vaccination and influenza vaccination rates, median parental age, parental vaccination rate, and parental education level were higher in vaccinated patients (p<0.001). Conclusion: Parents' concerns about safety and side effects were found to be the most important factors affecting vaccination success. Identification of the underlying causes of parental vaccine hesitancy will facilitate the development of effective vaccination strategies for potential future outbreaks.
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PURPOSE: To identify potential predictors of the disease course of systemic juvenile idiopathic arthritis (sJIA) at the time of diagnosis. METHODS: This retrospective observational study was conducted in patients diagnosed with sJIA in our hospital between April 2009 and October 2023. The relationship between the disease course of sJIA patients and demographic, clinical, laboratory findings and complications were analyzed. RESULTS: Of the 51 patients diagnosed with sJIA, 26 (51%) patients had monocyclic, 7 (13.7%) polycyclic and 18 (35.2%) persistent disease course. 3 (5.8%) patients had a persistent disease course with persistent arthritis developed flares with systemic manifestations during follow-up. The presence of arthritis, polyarticular involvement, and hip involvement at the time of diagnosis were associated with persistent disease course (p=0.009, p=0.003, p=0.003). Serositis and higher white blood cell and neutrophil counts at the time of diagnosis were associated with a monocyclic disease course (p=0.034, p=0.002, p=0.008). However, no significant correlation was found between macrophage activation syndrome (MAS) and disease course (p=1). CONCLUSIONS: Systemic JIA patients with polyarthritis and hip involvement at disease onset may develop a persistent course. Although MAS is an important complication of sJIA, its effect on the course of the disease was not found in this study.
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The purpose of this study was to compare the demographic and clinical characteristics of the groups with and without bDMARDs added to the treatment of persistent oligoarticular juvenile idiopathic arthritis (JIA) patients on methotrexate (MTX) and also to determine the predictors of adding bDMARDs to treatment. This study included 86 oligoarticular JIA patients on MTX. Patients were divided into two groups receiving MTX (n = 69) and MTX plus bDMARD (n = 17). Predictors of adding bDMARDs were investigated by comparing demographic, clinical features and laboratory findings. Gender, age at diagnosis, time elapsed from the onset of symptoms to diagnosis, and disease duration, the number and distribution of affected joint at the time of diagnosis were similar in both groups. The mean JADAS10 at the time of diagnosis were 18.8 ± 4.2 and 19.5 ± 6.4 in the MTX and MTX plus bDMARDs groups, respectively (p = 0.68). JADAS10 at 3rd and 6th month were significantly higher in patients on MTX plus bDMARDs (p = 0.001, p = 0.004, respectively). In multivariate analysis, the risk of adding bDMARD was shown to increase 1.24-fold (p = 0.004, 95% CI: 1.07-1.43) for each point increase on the JADAS 10 at 3rd months. The number (p = 0.64) or type (p = 0.18) of joint involvement at disease onset were not predictors of adding a bDMARD. CONCLUSION: JADAS10 indicating ongoing severe disease activity at 3rd and 6th months rather than baseline JADAS10 is associated with the addition of bDMARDs. WHAT IS KNOWN: ⢠Oligoarticular JIA patients have the best outcomes among JIA categories and respond favorably to first-line therapies such as non-steroidal anti-inflammatory drugs and intraarticular corticosteroid injections. ⢠Clinically inactive disease rates have increased with the widespread use of biological agents in oligoarticular JIA patients who have not responded to initial therapies. WHAT IS NEW: ⢠Approximately one-fifth of patients with persistent oligoarticular JIA on methotrexate may require the addition of a biological disease modifying anti-rheumatic drug during follow-up. ⢠The JADAS10 calculated at 3 and 6 months is a valuable tool to identify patients who should be added biological disease modifying anti-rheumatic drugs in persistent oligoarticular JIA.
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Antirreumáticos , Artritis Juvenil , Quimioterapia Combinada , Metotrexato , Humanos , Artritis Juvenil/tratamiento farmacológico , Artritis Juvenil/diagnóstico , Masculino , Femenino , Metotrexato/uso terapéutico , Niño , Antirreumáticos/uso terapéutico , Preescolar , Estudios Retrospectivos , Adolescente , Resultado del Tratamiento , Productos Biológicos/uso terapéuticoRESUMEN
OBJECTIVES: The aim of this study is to evaluate differences in school performance, school attendance, quality of life, and physical activity in adolescents with Familial Mediterranean fever (FMF) compared to healthy controls. METHODS: One hundred and twenty-nine patients with FMF and 154 healthy controls between 13 and 18 years were included in the study. Demographic, school performance (according to grade point average), school absenteeism, and type and frequency of exercise were recorded. Quality of life was evaluated with the Pediatric Quality of Life Inventory (PedsQL) 4.0. RESULTS: The mean age of FMF patients was 15.1 ± 2.7 years, and 69 patients (53.5%) were female. School performance was significantly higher in the control group compared to FMF patients (P < 0.001). In the control group, there were significantly higher participants who engaged in professional sports (P < 0.001). Patients with FMF had significantly lower self-reported PedsQL scores in school functioning, physical, and psychosocial health domains compared to those in the control group (P = 0.001, P < 0.001, and P = 0.028, respectively). CONCLUSIONS: FMF patients demonstrated lower school performance and quality-of-life scores compared to healthy controls. In addition to improving symptoms in chronic diseases, it is important to evaluate and improve the quality of life of patients in routine practice and to ensure psychosocial well-being.
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Fiebre Mediterránea Familiar , Niño , Humanos , Femenino , Adolescente , Masculino , Fiebre Mediterránea Familiar/diagnóstico , Calidad de Vida , Índice de Severidad de la Enfermedad , Ejercicio Físico , AutoinformeRESUMEN
BACKGROUND-AIM: To evaluate the effect of vitamin D supplementation on the frequency and duration of attacks in patients of PFAPA syndrome with low vitamin D levels. METHODS: This retrospective study comprised PFAPA patients with vitamin D deficiency/insufficiency between 2018 and 2023. The frequency and duration of PFAPA attacks before and after vitamin D supplementation were noted. RESULTS: Seventy-one patients were included. Of the 71 patients, 24 (33.8%) had vitamin D insufficiency, and 47 (66.2%) had vitamin D deficiency. In patients with vitamin D insufficiency, mean attack frequency and mean attack duration before vitamin D supplementation were 4.3 ± 1.9/year and 2.2 ± 1.6 days, respectively, while mean attack frequency and mean attack duration after vitamin D supplementation were 3.5 ± 2.7/year per year and 1.3 ± 0.9 days respectively (p = 0.2, p = 0.2, respectively). In patients with vitamin D deficiency, mean attack frequency and mean attack duration before vitamin D supplementation were 7.4 ± 2.1/year and 2.2 ± 1.6 days, respectively, while mean attack frequency and mean attack duration after vitamin D supplementation were 3.3 ± 2.4/year and 1.3 ± 0.9 days respectively (p < 0.01, p = 0.04, respectively). When the vitamin D level and the frequency of attacks were compared, the cut-off value of vitamin D was found to be 29.7 nmol/L. CONCLUSIONS: In PFAPA patients with low vitamin D levels, the frequency and duration of PFAPA attacks were reduced with vitamin D supplementation. Especially at vitamin D level cut-off > 29.7 nmol/L, the frequency of attacks reduced significantly.
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Linfadenopatía , Faringitis , Estomatitis Aftosa , Deficiencia de Vitamina D , Humanos , Estudios Retrospectivos , Vitamina D/uso terapéutico , Deficiencia de Vitamina D/complicaciones , Deficiencia de Vitamina D/tratamiento farmacológico , Estomatitis Aftosa/complicaciones , Estomatitis Aftosa/tratamiento farmacológico , Síndrome , Suplementos DietéticosRESUMEN
The purpose of this study was to evaluate physical activity (PA) and health-related quality of life (HRQOL) in children with oligoarticular juvenile idiopathic arthritis (JIA) in remission in comparison with healthy peers and to determine the disease-related factors affecting PA levels. This study was conducted with 50 oligoarticular JIA patients in remission and 50 healthy peers between 9 and 14 years. Demographic and clinical characteristics, laboratory parameters, and treatments were noted from electronic medical records. HRQOL was assessed with the Pediatric Quality of Life Inventory (PedsQL). PA was evaluated with the Physical Activity Questionnaire for Children (PAQ-C). Oligoarticular JIA patients had significantly lower self-reported median PedsQL scores in the domains of school functioning and social functioning compared to the control group (67.5 (10) vs. 75 (25), p = 0.001 and 70 (15) vs. 85 (26.3), p < 0.001, respectively). The median PAQ-C score was 2.6 (1.1) in patients with JIA and 3 (0.9) in their healthy peers (p = 0.02). The PAQ-C score was 2.8 (1.2) in patients < 8 years at the disease onset and 2.3 (1) in those aged ≥ 8 years (p = 0.022). There was no significant difference in the number of affected joints, type of affected joint, MTX and biologic agent treatment, and remission with or without drugs with the total score of the PedsQL and PAQ-C. All PedsQL domains were positively correlated with the PAQ-C. Conclusion: Oligoarticular JIA patients demonstrated lower PA and HRQOL scores compared to healthy controls despite favorable disease control. What is Known: ⢠Oligoarticular JIA has fewer functional limitations and disabilities compared to other JIA subtypes. ⢠As JIA can affect all aspects of a child's life, there is a need to improve the quality of life related to the disease. What is New: ⢠It should be considered that patients with oligoarticular JIA may show lower PA and HRQOL scores compared to healthy controls despite favorable disease control. ⢠Since there may be a relationship between PA and HRQOL, factors that may affect PA should be investigated to provide a holistic approach to JIA treatment.
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Artritis Juvenil , Calidad de Vida , Niño , Humanos , Artritis Juvenil/tratamiento farmacológico , Estado de Salud , Ejercicio Físico , Encuestas y CuestionariosRESUMEN
AIM: To evaluate the treatment response to compressed colchicine tablets in familial Mediterranean fever (FMF) patients with resistance or intolerance to coated colchicine. The secondary aim was to determine the demographic and clinical characteristics of responders to compressed colchicine. METHODS: We retrospectively reviewed the medical records of 1574 pediatric patients with FMF treated at Ankara Bilkent City Hospital. Sixty-one patients did not respond to coated colchicine and were switched to compressed colchicine. In these patients, the number of attacks and the International Severity Score for FMF (ISSF) during the 6 months before and 3, 6, 9, 12, and 24 months after switching from coated colchicine to compressed colchicine were recorded. RESULTS: Twelve of 61 patients (19.7%) who were switched to compressed colchicine due to intolerance responded to treatment. Of the 49/61 patients (80.3%) who were switched due to uncontrolled attacks and persistent subclinical inflammation, 25 responded to treatment. The frequency of attacks and ISSF decreased after switching. At the end of the two-year follow-up, 42 patients responded to compressed colchicine, and 19 patients received compressed colchicine plus interleukin-1-targeting drugs. CONCLUSIONS: Compressed colchicine was shown to be a useful treatment option before initiating biological agents in non-responders to coated colchicine, especially those with side effects.
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Colchicina , Fiebre Mediterránea Familiar , Humanos , Niño , Colchicina/uso terapéutico , Fiebre Mediterránea Familiar/tratamiento farmacológico , Fiebre Mediterránea Familiar/inducido químicamente , Fiebre Mediterránea Familiar/complicaciones , Estudios Retrospectivos , Interleucina-1RESUMEN
OBJECTIVES: Our study aimed to evaluate the relationship of small joint involvement with demographic, clinical, and laboratory findings and to determine its possible effects on prognosis. METHODS: This retrospective observational study was conducted in patients diagnosed with oJIA in the pediatric rheumatology department of our hospital between April 2009-September 2022. The relationship between small joint involvement and demographic, clinical, laboratory findings and prognosis were investigated by statistical methods with the data recorded from the medical records of oJIA patients. RESULTS: Of the 198 patients diagnosed with oJIA, small joint involvement was observed in a total of 20 (10%) patients, 11 (5.5%) at the time of diagnosis, and 9 (4.5%) during the follow-up period. The frequency of small joint involvement in extended oJIA was significantly higher than in persistent oJIA (p=0.001). Patients with small joint involvement had significantly higher ESR and CRP values at admission (p=0.047, p=0.038) and the JADAS at 3, 6, and 12 months (p=0.001, p=0.001, p=0.018). The need for cDMARDs and bDMARDs was significantly higher in patients with small joint involvement (p=0.001, p=0.001). CONCLUSIONS: oJIA patients with small joint involvement may have higher acute phase reactants at diagnosis, a more extended course and active disease in follow-up, and the need for treatment escalation.
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OBJECTIVE: The aim of this study is to evaluate the clinical, laboratory, and radiological findings and prognosis of patients with adenosine deaminase 2 deficiency (DADA2) and to highlight the conditions that DADA2 should be considered in the differential diagnosis in patients with neurological findings. METHODS: A case series of six DADA2 patients was presented in this retrospective, descriptive study. Clinical and laboratory data, treatment protocols, and prognosis of the patients were recorded. A diagnosis of DADA2 was established by ADA2 enzyme activity assay and/or ADA2 gene sequencing. RESULTS: Six patients with DADA2 were included in the study. The median age at symptom onset was 6.5 years (range 3.5-13.5 years). The median time to diagnosis from the initial presentation was 9 (3-72) months. Consanguinity was present in the families of 4 cases. The skin, nervous system, and musculoskeletal system were the most commonly involved systems. Vasculitis mimicking polyarteritis nodosa (PAN) was the predominant phenotype (n=4) in our case series. Four patients with PAN-like features had neurological involvement. Ischemic strokes were found in 3 patients, cranial nerve palsy in 2 patients, and seizures in 2 patients. The CECR1 gene was analyzed in all patients. We analyzed plasma ADA2 enzyme activity only in one patient. Anti-tumor necrosis factor (TNF)-α therapy was initiated. Inflammation was suppressed and remission was achieved in all patients. CONCLUSION: DADA2 should be considered in patients with PAN-like disease, a history of familial PAN/vasculitis, early-onset strokes/neurological involvement with systemic inflammation. Furthermore, anti-TNF-α therapy appears to be beneficial for the treatment of DADA2.
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BACKGROUND: The aim of this study was to evaluate the role of the systemic immune inflammation index (SII), C-reactive protein/albumin ratio (CAR), the monocyte/lymphocyte ratio (MLR), and the neutrophil/lymphocyte ratio (NLR) in predicting disease severity, treatment, and prognosis in multisystem inflammatory syndrome in children (MIS-C). METHODS: This medical record review retrospectively evaluated the clinical and laboratory findings of 191 MIS-C patients followed in the Department of Pediatric Rheumatology at Ankara City Hospital, Turkey. The patients were grouped by disease severity: mild, moderate, and severe. SII, CAR, MLR, and NLR were calculated for each group. RESULTS: All patients had fever at the time of admission; 153 (80.1%) had gastrointestinal tract involvement, 74 (38.7%) had rash, 63 (33%) had conjunctivitis, 107 (56%) had cardiac involvement, 32 (15.6%) had renal involvement, and 143 (74.9%) had hematological involvement. According to logistic regression analysis, SII, NLR, MLR, and CAR were found to be predictive indexes for disease severity, need for intensive care, need for inotropes, and anakinra treatment in MIS-C. The cut-off values of ≥1605.3 for SII, ≥9.1 for NLR, and ≥3.9 for CAR increased the risk of severe disease by 3.4, 7.1, and 5.7 times, respectively. CONCLUSION: NLR, SII, MLR, and CAR are effective and useful for predicting the severity of MIS-C, the need for intensive care, and the need for anakinra treatment.
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Proteína Antagonista del Receptor de Interleucina 1 , Síndrome de Respuesta Inflamatoria Sistémica , Niño , Humanos , Estudios Retrospectivos , Síndrome de Respuesta Inflamatoria Sistémica/diagnóstico , Síndrome de Respuesta Inflamatoria Sistémica/terapia , Inflamación , Gravedad del Paciente , Neutrófilos , LinfocitosRESUMEN
The aim of this study was to evaluate the predictors of relapse in patients with oligoarticular juvenile idiopathic arthritis (oJIA) who achieved clinical remission off medication. This retrospective observational study was conducted between June 2009 and July 2022 in 126 patients with oJIA who achieved remission off medication. The relationships between relapse status and demographic, clinical and laboratory findings, and treatment details were evaluated using electronic medical records. Of the 126 oJIA patients who achieved remission off medication, 85 (67.5%) were female. Relapse occurred in 31 patients (24.6%) with remission off medication after a median of 18 months (IQR 7-26). No statistically significant relationship was found between gender, age at diagnosis, oJIA subtype, number of joints, ANA, ESR, CRP level, initial Juvenile Arthritis Disease Activity Score and relapse in oJIA patients who achieved remission off medication (p = 0.66, p = 0.25, p = 1, p = 0.54, p = 0.29, p = 0.59, p = 0.95 and p = 0.52, respectively). There was a statistically significant relationship between the number of intraarticular corticosteroid injections (IACIs) and relapse (p = 0.01). Patients who underwent IACI 2-3 times had more relapses than those who never underwent IACI and those who underwent IACI only once (p = 0.01, p = 0.02, respectively). A relationship was found between the length of follow-up and relapse in patients with oJIA who achieved remission off medication (p = 0.035). Conclusion: In oJIA patients who achieve remission off medication, the probability of relapse increases in patients who need ≥ 2 IACI during the period until remission. The length of follow-up period is associated with the probability of relapse. What is Known: ⢠Approximately one-fourth of oJIA patients who are in remission off medication have relapse. ⢠There is a need for markers that can predict the risk of relapse in oJIA patients who achieve remission on or off medication. What is New: ⢠The possibility of relapse should be considered in patients with oJIA who need ≥ 2 IACIs until achieving remission off medication. ⢠The relapse rate may increase as the follow-up period prolongs in patients who achieve remission off medication.
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Artritis Juvenil , Humanos , Femenino , Masculino , Artritis Juvenil/diagnóstico , Artritis Juvenil/tratamiento farmacológico , Estudios Retrospectivos , Corticoesteroides/uso terapéutico , Enfermedad Crónica , Recurrencia , Resultado del TratamientoRESUMEN
The frequency of vitamin D deficiency in healthy children is relatively high. Moreover, vitamin D supplementation in children is under the requested levels. The aim of this study is to determine the frequency of vitamin D deficiency and the factors that influence vitamin D levels in healthy children. During the study period, 3368 vitamin D levels of healthy children, aged 0-18 years, were evaluated retrospectively. Vitamin D levels were categorized as deficiency (< 12 ng/ml), insufficiency (12-20 ng/ml), and sufficiency (> 20 ng/ml). The prevalence of vitamin D deficiency and insufficiency was found to be 18-24.9% in healthy children, respectively. It was found that the frequency of vitamin D deficiency increased with age. In addition, the most severe and highest risk group for vitamin D deficiency were adolescent girls. Moreover, being in the winter or spring season and living in the north of the 40th parallel are the other risk factors for vitamin D deficiency. CONCLUSION: This study showed that vitamin D deficiency still remains a major problem for healthy children and daily supplementation of vitamin D is mandatory. Prophylactic vitamin D supplementation and adequate sunlight exposure should be provided for all children, in particular healthy adolescents. In addition, future studies may focus on screening for vitamin D status in children who did not receive vitamin D supplementation. WHAT IS KNOWN: ⢠Vitamin D is an essential component in bone metabolism. Seasonality, age, sex, dark skin pigmentation, and limited exposure to sunlight are causes of vitamin D deficiency. ⢠The World Health Organization has drawn attention to this increased frequency, recommending lifelong, regular vitamin D prophylaxis. WHAT IS NEW: ⢠The frequency of vitamin D deficiency and insufficiency was found to be 42.9% in healthy children and the frequency significantly increased with age. ⢠There were almost no cases of prophylactic vitamin D usage in adolescent group which are at the highest risk.
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Deficiencia de Vitamina D , Vitamina D , Niño , Adolescente , Femenino , Humanos , Recién Nacido , Lactante , Preescolar , Vitamina D/uso terapéutico , Estudios Retrospectivos , Deficiencia de Vitamina D/diagnóstico , Deficiencia de Vitamina D/epidemiología , Deficiencia de Vitamina D/etiología , Vitaminas , Factores de Riesgo , Prevalencia , Estaciones del AñoRESUMEN
BACKGROUND: The Eurofever/the Pediatric Rheumatology International Trials Organization (PRINTO) classification criteria for familial Mediterranean fever (FMF) include a combination of clinical symptoms and genotype. The pathogenicity of gene variants associated with FMF is categorized by the International Study Group for Systemic Autoinflammatory Diseases (INSAID) classification criteria. OBJECTIVE: The aim of this study was to evaluate the real-life impact and usefulness of the Eurofever/PRINTO classification criteria and the INSAID classification criteria in patients with FMF and their impact on treatment management. METHODS: In this medical records review study, the files of FMF patients who met the Eurofever/PRINTO classification criteria were reviewed. The MEFV (MEditerranean FeVer) variants were grouped according to the INSAID classification criteria. RESULTS: Of the 1062 patients, the female-to-male ratio was 1:1.01. In group 1, there were 150 patients (14.1%) who met the clinical criteria. Group 2 consisted of 912 patients (85.9%) who met the criteria according to genetic variants. The mean ages at symptom onset in groups 1 and 2 were 5.6 ± 3.8 and 1.5 ± 1.2 years, respectively ( p = 0.024). Whereas the mean annual attack frequency was 2.7 ± 3.1/year in group 1, it was 4.1 ± 2.3/year in group 2 ( p = 0.04). The pathogenic variant was higher in the colchicine-resistant group compared with the responders ( p = 0.12). CONCLUSIONS: The Eurofever/PRINTO classification criteria may provide a new perspective on the diagnosis and clinical follow-up of FMF patients. Patients with a pathogenic variant who meet the Eurofever/PRINTO classification criteria including genetic variables have earlier onset of disease and more frequent attacks than those who meet the criteria including clinical variables. These patients need regular and closer follow-ups in terms of attack frequency, colchicine dose adjustment, and colchicine resistance.
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Fiebre Mediterránea Familiar , Niño , Humanos , Masculino , Femenino , Fiebre Mediterránea Familiar/diagnóstico , Fiebre Mediterránea Familiar/tratamiento farmacológico , Fiebre Mediterránea Familiar/genética , Colchicina/uso terapéutico , Genotipo , Pirina/genéticaRESUMEN
OBJECTIVE: To compare enthesitis-related arthritis (ERA) patients with active and inactive disease at 6 months and define baseline predictors for disease inactivity. In addition, to evaluate the demographic, clinical, and laboratory characteristics of ERA patients and to identify the real-life impact of the Juvenile Spondyloarthritis Disease Activity Index (JSpADA) in predicting active disease in ERA. METHODS: This medical record review study was conducted with 56 patients who were diagnosed with ERA at our clinic between June 2009 and June 2022. Demographic and clinical characteristics, laboratory parameters, treatment, and JSpADA were recorded. RESULTS: The patients were divided into 2 groups as active (n = 34) and inactive (n = 22) according to their disease activity at month six. Sex, age at diagnosis, number and type of affected joints, and presence of sacroiliitis were similar in both groups. There was no difference in baseline erythrocyte sedimentation rate, but there was a significant difference in erythrocyte sedimentation rate at the third month ( p = 0.52 and p = 0.018, respectively). The median JSpADA values at disease onset were 3.5 (interquartile range [IQR], 3.0-4.5) and 3.3 (IQR, 2.5-4.0) in the active and inactive groups, respectively ( p = 0.27). At the third month, the median JSpADA values were 1.5 (IQR, 0.5-2.1) in the active group and 0.5 (IQR, 0.5-1.5) in the inactive group ( p = 0.037). The cutoff value for JSpADA at the third month for active disease persisting at the month six was determined as 1 point (area under the curve, 0.662 ± 0.06; p = 0.042; 95% confidence interval, 0.51-0.80) by receiver operating characteristic curve analysis. CONCLUSION: In ERA patients, a persistently high JSpADA value at follow-up is a predictive factor for active disease at the sixth month.
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Artritis Juvenil , Sacroileítis , Espondiloartritis , Humanos , Estudios Retrospectivos , Artritis Juvenil/diagnóstico , Artritis Juvenil/tratamiento farmacológico , Sacroileítis/diagnóstico , Sacroileítis/etiología , Índice de Severidad de la Enfermedad , Espondiloartritis/diagnósticoRESUMEN
OBJECTIVE: The purpose of this study is to investigate the causes and outcomes of switching biological agents in juvenile idiopathic arthritis (JIA) patients using biological agents and compare the characteristics of patients whose biological agents are switched and those whose are not. METHODS: This medical records review study was conducted with 128 patients who were diagnosed with JIA at our clinic between January 2009 and January 2022 and were receiving biologic agents. Factors affecting the biologic agent switching were investigated. RESULTS: The JIA subtype with the most frequent switching in biological agents was systemic JIA (n = 13, 40.6%). Systemic JIA was followed by rheumatoid factor-negative polyarticular JIA and persistent oligoarticular JIA with 5 patients (15.6%), extended oligoarticular JIA and enthesitis-related JIA with 3 patients (9.3%), rheumatoid factor-positive polyarticular JIA with 2 patients (6.2%), and undifferentiated JIA with 1 patient (3.1%). Among the patients, 32 (25%) patients had their biological agent switched once, and 5 (3.9%) had theirs switched twice. The most frequently used biological agent was etanercept (n = 76, 59.3%), whereas the most frequently observed cases of biological agent switching were from an anti-TNF agent to another anti-TNF agent (40.6%). The reason for switching was unresponsiveness to the agent in 22 patients (68.8%), adverse effects in 6 patients (18.7%), drug intolerance in 1 patient (3.1%), and other reasons in 3 patients (9.3%). CONCLUSIONS: The most frequently used biological agent was etanercept; the most frequent cases of biological agents switching were from an anti-TNF agent to another anti-TNF agent.
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Antirreumáticos , Artritis Juvenil , Humanos , Artritis Juvenil/diagnóstico , Artritis Juvenil/tratamiento farmacológico , Etanercept/efectos adversos , Factores Biológicos/efectos adversos , Antirreumáticos/efectos adversos , Factor Reumatoide , Inhibidores del Factor de Necrosis Tumoral/uso terapéuticoRESUMEN
To evaluate the general characteristics of pediatric Behçet's disease (BD) patients with thrombus and to present the clinical features, treatment responses and prognosis of patients with intracardiac thrombus. The clinical characteristics and outcomes of 15 patients with thrombus among 85 pediatric BD patients followed in the Department of Pediatric Rheumatology were evaluated retrospectively. Of the 15 BD patients with thrombus, 12 (80%) were male, 3 (20%) were female. The mean age at diagnosis was 12.9 ± 1.1 years. Thrombus was present at the time of diagnosis in 12 patients (80%), while thrombus developed in three patients within the first three months after diagnosis. The most common site of thrombus was the central nervous system (n = 9, 60%), followed by deep vein thrombus (n = 6, 40%) and pulmonary artery thrombus (n = 4, 26.6%). Three male patients (20%) developed intracardiac thrombus. The overall intracardiac thrombus rate in the 85 patients was 3.5%. Two of the three patients had thrombus in the right, and one had thrombus in the left heart cavity. In addition to steroids, 2 of the 3 patients received cyclophosphamide, while the patient with thrombus localized in the left heart cavity was given infliximab. In the follow-up, the two patients with thrombus in the right heart cavity were switched to infliximab because of resistance to cyclophosphamide. Complete resolution was observed in 2 of the 3 patients on infliximab; a significant reduction in the thrombus of the other patient was achieved. Intracardiac thrombus is a rare presentation of cardiac involvement in BD. It is usually observed in males and in the right heart. Although steroids and immunosuppressive agents such as cyclophosphamide are recommended as first-line treatment, favorable outcomes can be achieved with anti-TNFs in resistant cases.
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Síndrome de Behçet , Arteria Pulmonar , Trombosis , Síndrome de Behçet/complicaciones , Síndrome de Behçet/tratamiento farmacológico , Humanos , Masculino , Femenino , Niño , Adolescente , Trombosis/diagnóstico , Trombosis/etiología , Estudios Retrospectivos , Arteria Pulmonar/diagnóstico por imagen , Angiografía por Tomografía Computarizada/métodos , Esteroides/uso terapéutico , Ciclofosfamida/uso terapéutico , Infliximab/uso terapéutico , Inmunosupresores/uso terapéutico , Resultado del TratamientoRESUMEN
BACKGROUND: The purpose of this study was to compare the demographic, clinical and laboratory characteristics of patients with enthesitis-related arthritis (ERA), familial Mediterranean fever (FMF) and inflammatory bowel disease (IBD), which are inflammatory diseases that may develop sacroiliitis. Thus, it was aimed to reveal various findings that may indicate primary disease in patients with sacroiliitis. METHODS: Pediatric patients aged 6-18 years, who were being followed with a diagnosis of ERA (n = 62), FMF (n = 590), and IBD (n = 56) over the period 2013-2021 were included in the study. Sacroiliitis (n = 55) was diagnosed by magnetic resonance imaging of the sacroiliac joint, obtained from clinically suspected patients. RESULTS: Sacroiliitis was detected in 54.8% of ERA patients, 2.3% of FMF patients, and 12.5% of IBD patients. The mean follow-up period was 4.1 ± 2.8 years (10 months-8 years) for the entire study group. The most common MRI finding for sacroiliitis was bone marrow edema. Peripheral joint involvement (73.5%) and HLA B27 positivity (64.7%) was significantly higher in ERA patients, and ERA was diagnosed more frequently in patients presenting with sacroiliitis. Non-steroidal anti-inflammatory drugs (NSAIDs) were the first choice of treatment agent when sacroiliitis developed in all three patient groups. CONCLUSIONS: The clinical and laboratory findings of ERA, FMF and IBD can sometimes be intertwined or can even coexist. Treatment may differ depending on the disease associated with sacroiliitis, although NSAIDs may be used in the first-line treatment of all three diseases. Sacroiliitis patients with HLA B27 positivity and peripheral arthritis may need to be addressed as ERA.
Asunto(s)
Artritis Juvenil , Fiebre Mediterránea Familiar , Enfermedades Inflamatorias del Intestino , Sacroileítis , Humanos , Niño , Sacroileítis/diagnóstico , Sacroileítis/tratamiento farmacológico , Antígeno HLA-B27 , Diagnóstico Diferencial , Artritis Juvenil/complicaciones , Artritis Juvenil/diagnóstico , Artritis Juvenil/tratamiento farmacológico , Imagen por Resonancia Magnética/métodos , Antiinflamatorios no Esteroideos/uso terapéutico , Fiebre Mediterránea Familiar/diagnóstico , Enfermedades Inflamatorias del Intestino/diagnósticoRESUMEN
AIM: To identify the risk factors associated with intussusception in children with immunoglobulin A vasculitis (IgAV)-gastrointestinal (GI) tract involvement and to evaluate the outcomes of medical treatment and surgical intervention and the course of patients with intussusception. METHODS: This retrospective study was conducted in 157 patients under 18 years of age who were followed up with the diagnosis of IgAV-GI tract involvement between January 2015 and September 2022. The characteristics of the patients who developed intussusception were evaluated in detail. RESULTS: One hundred and fifty-seven patients with GI tract involvement were included in the study. The mean age of patients with IgAV-GI tract involvement was 8.7 ± 3.7 years. The female-to-male ratio was 1:1.5. Intussusception was detected in 14 patients (8.9%). Two patients (14.3%) underwent surgery, and the remaining 12 patients (85.7%) had their medical therapy intensified. Patients with GI tract involvement were divided into two groups as with (n = 14) and without (n = 143) intussusception. There was a statistically significant difference between the groups in the time from the onset of the first symptom of IgAV to the onset of steroids (P = 0.001). There were no statistically significant differences between the groups in age at onset of IgAV, gender distribution, erythrocyte sedimentation rate and C-reactive protein levels. CONCLUSIONS: The time from the onset of the first symptom of IgAV to the start of steroids is a risk factor for the development of intussusception in patients with IgAV-GI tract involvement. In these patients, medical treatment usually reduces intussusception without the need for surgical intervention.
Asunto(s)
Vasculitis por IgA , Intususcepción , Niño , Humanos , Masculino , Femenino , Adolescente , Preescolar , Estudios Retrospectivos , Intususcepción/etiología , Intususcepción/terapia , Inmunoglobulina A , Vasculitis por IgA/complicaciones , Vasculitis por IgA/diagnóstico , Factores de RiesgoRESUMEN
OBJECTIVE: The present study aimed to compare the demographic, clinical, and laboratory characteristics of patients with pediatric Behçet disease (BD) with and without thrombosis to elucidate the factors that may contribute to the development of thrombosis. METHODS: This observational, descriptive, medical records review study included patients with BD (n = 85) who were diagnosed at age younger than 16 years at our clinic between 2010 and 2022. The demographic, clinical, and available laboratory data of patients with and without thrombosis were compared. The potential risk factors for the development of thrombosis were evaluated with multivariable logistic regression analysis. RESULTS: Central venous sinus thrombosis was the most common type of thrombosis. Thrombosis was significantly more common in male patients ( p = 0.002), and regression analysis revealed that being male was a risk factor for developing thrombosis. Genital ulcers were less common in patients with thromboses. Patients with thrombosis had higher erythrocyte sedimentation rates, C-reactive protein, leukocyte, and neutrophil counts, as well as antinuclear antibody positivity. In contrast, mean platelet volume and lymphocyte counts were significantly lower in patients with thrombosis. According to the logistic regression analysis, erythrocyte sedimentation rate value >17 mm/h was a risk factor for developing thrombosis (odds ratio, 1; confidence interval, 1.1-1.8; p = 0.012). CONCLUSIONS: Male sex has been associated with an increased risk of thrombosis in children with BD. Inflammatory parameters may serve as predictive factors for thrombosis in pediatric BD.